Nephrotic syndrome in autosomal dominant polycystic kidney disease.

Journal of the American Society of Nephrology : JASN
G ContrerasC A Vaamonde

Abstract

Urinary protein excretion is generally less than 1 g/24 h in autosomal dominant polycystic kidney disease (ADPKD), and the association of the nephrotic syndrome with this condition is considered rare. A patient with ADPKD associated with nephrotic-range proteinuria is described. She exhibited a relatively rapid impairment of her renal function. An open renal biopsy revealed focal segmental glomerulosclerosis (FGS) with features consistent with secondary FGS. Twenty-one patients with ADPKD and nephrotic syndrome were retrieved from the literature. Fourteen of them (including this case) had a histopathologic evaluation, and FGS was the dominant diagnoses (five patients). Next in frequency were minimal-change disease and membranous nephropathy, with two patients each. Five other patients had a variety of diagnoses. Thus, it is difficult to ascertain if these associations are coincidental or represent a specific pathogenetic relationship. The evaluation of the data also suggests that the presence of proteinuria and nephrotic syndrome accelerates the course of ADPKD toward ESRD.

Citations

May 11, 2002·Scandinavian Journal of Urology and Nephrology·C BosmanR Boldrini
Jun 23, 2006·Clinical and Experimental Nephrology·Toru HiuraFumitake Gejyo
Feb 24, 2007·International Urology and Nephrology·Fuat SarRumeyza Kazancioglu

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